Leontiasis Research Articles

Leontiasis Ossea in Hyperparathyroidism Secondary to Inadequate ESKD Management

Leontiasis Ossea in Hyperparathyroidism Secondary to Inadequate ESKD Management

Uremic leontiasis ossea: A pictorial review of a rare complication of renal osteodystrophy

Uremic leontiasis ossea: A pictorial review of a rare complication of renal osteodystrophy

Uremic Leontiasis Ossea Secondary to Hyperparathyroidism in Three Patients with End Stage Renal Disease

Renal osteodystrophy is a common complication referring to the skeletal changes associated with the phosphocalcic disorders of chronic uremia in patients with end-stage kidney disease. Uremic leontiasis ossea (ULO) is a rare and severe form of renal osteodystrophy with characteristic overgrowth of the cranial vault and facial bones. Computed tomography (CT) findings are particularly characteristic and include serpiginous tunneling within the maxillofacial bones and cortical bone resorption. The recognition of its radiological appearance and abrupt management are essential to avoid its devastating esthetic and functional impairments. Today's better medical facilities have improved hemodialysis methods, and meticulous patient follow‐up has decreased the incidence of leontiasis. In this paper, we report three rare cases of patients with end stage renal failure who presented with multiple face swellings and were diagnosed with ULO.

Leontiasis Ossea about a Case Treated at Chu Owendo

Introduction: Leontiasis ossea is the most severe bone complication of the maxillae of chronic renal failure. The objective of this work is to determine the clinical, paraclinical and therapeutic aspects of this condition. Observation: A 42-year-old patient with chronic anuric terminal renal failure dialysis for 12 years had consulted for multiple maxillo-mandibular swelling evolving for 2 years deforming the face with discomfort to chewing. The biological assessment was: PTH: 5252 pg/mL, Ca2+: 2.86 mmol/L, free T3: 4.16 pmol/L, free T4: 6.55 pmol/L, TSH: 3.13 U/mL, serum protein: 61 g/L, Creatinine: 835 μmol/L. The maxillofacial CT noted heterogeneous diffuse thickening of the cranial vault and bones of the facial massif evoking leontiasis ossea with right mandibular brown tumor. The biopsy diagnosed fibrous gingival epulis. The patient had undergone modeling surgery, under general anesthesia after dialysis, of the right mandibular tumor whose histological result was Leontiasis ossea. Conclusion: Treatment is modeling surgery under hemostatic control.

Abstract #1148697: Severe Facial Disfiguration due to Uremic Leontiasis Ossea from Underlying Secondary Hyperparathyroidism

Renal osteodystrophy is a common complication of end-stage renal disease (ESRD) patients. Rarely, it can be associated with uremic leontiasis ossea (ULO) that is characterized by progressive enlargement of the facial bones and is thought to be secondary to uncontrolled secondary hyperparathyroidism (sHPTH) in the setting of ESRD. Few case reports have been described in the literature with our case appearing to be the most severe. Due to the rarity of this disease, there is no current standard of treatment.

Three Rare Concurrent Complications of Tertiary Hyperparathyroidism: Maxillary Brown Tumor, Uremic Leontiasis Ossea, and Hungry Bone Syndrome.

A 48-year-old woman in her 40’s with end-stage renal disease and tertiary hyperparathyroidism (HPT) presented for a rapidly progressive maxillary tumor. Initial workup was notable for elevated intact parathyroid hormone (PTH) and diffuse thickening of skull and facial bones on computed tomography, and maxillary tumor biopsy with multinucleated giant cells. She underwent subtotal parathyroidectomy (with removal of a parathyroid adenoma and 2 hyperplastic glands) and partial resection of maxillary brown tumor. The patient’s post-operative course was complicated by hungry bone syndrome, with hypocalcemia refractory to aggressive calcium repletion. Teriparatide (recombinant PTH) was utilized with rapid resolution of hypocalcemia. To our knowledge, this is the first case of maxillary brown tumor in tertiary HPT to be reported in the USA. This case also supports teriparatide as a novel therapeutic for hungry bone syndrome refractory to aggressive calcium repletion.

Uremic leontiasis ossea inducing respiratory failure in a patient with stage 5 chronic kidney disease.

Uremic leontiasis ossea inducing respiratory failure in a patient with stage 5 chronic kidney disease.

Infantile malignant osteopetrosis (Leontiasis ossea) in a mid-19th c. French skull

Abstract Introduction Autosomal recessive osteopetrosis (ARO) is a rare genetic disorder that is also called “malignant osteopetrosis” because it typically manifests within the first few months after birth and later manifests severe complications (blindness, deafness, facial palsy, dental caries, hypocalcemia) and medullary haematopoetic disorders. The aim of this study is to describe lesions and conclude hypothetical clinical symptoms by performing a CT-scan analysis on an early 19th century human skull preserved in the Dupuytren museum collection (Paris). Materials and methods The dried skull was examined and described using classical methods of paleopathology. We conducted additional research using historical data on the skull and a CT scan was performed. Results Screening of the archives from the museum allowed us to estimate the skull's origin as somewhere between 1850 and 1900, with an estimated age of six years. Several ducts and foramens of the skull and the facial bones were obstructed as a result of this disease, clinically corresponding to diffuse neurological (and also vascular) deficits. This devastating pathology led to many symptoms stemming from brain tissue damage and cranial nerve deficits, leading to the inevitable death of this child. These symptoms include headache, intracranial hypertension with vomiting, blindness, exophthalmos, deafness, oral breathing, eating disorder (suffocation), facial palsy, balance disorder (excessive weight, vestibulo-cochlear and cerebellar involvement). Conclusion The study of this specimen from the 19th century confirms that the six-year old patient suffered from malignant osteopetrosis. CT-scan allowed confirmation of the diagnosis, as well as a complete description of the disease, and highlight a possible associated anaemia.

Leontiasis ossea; a rare presentation of secondary hyperparathyroidism

Leontiasis ossea is a rare presentation of renal osteodystrophy with few reports in literature. It causes severe skeletal deformity in the cranial bones. This study presents a report on a patient with advanced hyperparathyroidism with skeletal changes compatible with leontiasis ossea. The patient was a 34-year-old man with end-stage renal disease, uncontrolled hypertension, and dyspnea who was referred to our hospital. Physical examinations revealed: toggled speech (nasal speech), mouth breathing and facial change, saddle nose, nares widening, increased interdental space and mandibular enlargement. His serum intact parathyroid hormone (iPTH) level was 3199 pg/mL, Paranasal sinuses CT scan showed a significant expansion of the mandibular, maxillary and skull bones. Parathyroid sonography reported two severe hypertrophied parathyroid glands. The patient underwent total parathyroidectomy as a result of symptomatic bone involvement.

Uremic Leontiasis Ossea: A Rare Presentation of Chronic Renal Failure Secondary to Hyperparathyroidism

Facial skeletal changes associated with hyperparathyroidism assume three radiographic patterns: osteitis fibrosa cystica, fibrous dysplasia, and leontiasis ossea. The 3rd pattern is unique to renal osteodystrophy. Renal osteodystrophy frequently affects the spine, ribs, long bones, and skull. Findings of renal osteodystrophy in facial and cranial bones are rare. However, it’s most severe osseous complication is characterized by massive thickening of the cranial vault and facial bones, called uremic leontiasis ossea (ULO), with only few cases reported in the literature. The uremic leontiasis ossea causes significant aesthetic and functional changes. It is important to recognize features of leontiasis ossea, as it may result in life threatening upper airway obstruction and compressive cranial neuropathy while after parathyroidectomy, facial changes can be stabilized or improved mildly. We report a case of uremic leontiasis ossea with a history of gradual enlargement of the facial bones over a period of one year. Significant hypertrophy of the maxilla and clavarial bone is most significant CT finding with serpiginous tunneling within the bone and poor visualization of the cortical bone. Nuclear medicine scans are also useful for demonstrating parathyroid adenoma. Ultimately, the diagnosis of uremic leontiasis ossea can be made non-invasively through a combination of clinical parameters and imaging findings, as described in this article.

Whole body imaging of a leontiasis ossea patient with Paget disease

Whole body imaging of a leontiasis ossea patient with Paget disease

Sixty-Two-Year-Old Male Suffering From Uremic Leontiasis Ossea Caused by Severe Secondary Hyperparathyroidism.

ABSTRACTSecondary hyperparathyroidism (SHPT) is a long‐term complication of chronic kidney disease–mineral and bone disorder (CKD‐MBD). SHPT is characterized by hyperplasia of the parathyroid glands and abnormal secretion of parathyroid hormones (PTH), calcium and phosphorous metabolic disorders, renal osteodystrophy, vascular and soft tissue calcification, malnutrition, and other multiple system complications, which can seriously affect the quality of life of the patient and increase the risk of cardiovascular disease and mortality rate. Uremic leontiasis ossea (ULO) is a medical condition only rarely encountered clinically. SHPT causes craniofacial bone deformity accompanied by lesions of the nerve, cardiovascular, respiratory, bone, or other systems within the body. The case discussed here is related to severe SHPT. A 62‐year‐old male patient was suffering from leontiasis ossea, pectus excavatum, vascular calcification, spontaneous bone fractures, and lower limb deformities. He was undergoing hemodialysis and given total parathyroidectomy (TPTX) with autotransplantation (AT). We further analyzed the multivariate therapeutic effects of TPTX on this patient in order to provide clinical data for standardized treatment of individuals with CKD‐MBD. © 2018 The Authors JBMR Plus published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research.

Paget Disease of the Bone Presenting as Leontiasis Ossea

Clinical-Medical Image: Paget disease of the bone is a chronic disorder of unknown etiology that typically results in enlarged, deformed bones due to abnormal and excessive bone remodeling. The disease which can be monostotic or polyostotic and is characterized initially by an increase in bone resorption, followed by a disorganized and excessive formation of bone, leading to pain, fractures, and deformities. The classically described radiological appearances are of expanded bone with a coarsened trabecular pattern, but it can present differently according to the disease stage: in the active or osteolytic phase, aggressive bone resorption and lytic appearance are the hallmarks while on the quiescent and inactive stage there is a predominance of cortical thickening and sclerosis, and finally, it can have a mixed pattern presentation. The pelvis, spine, skull, and proximal long bones are most frequently affected. We depict an exuberant case of this disease with craniofacial involvement resulting in the historical eponym of Leontiasis Ossea (lion mask face) (Figures 1 and 2).

Visual Vignette

Case Presentation: An 84-year-old woman presents to the clinic with a primary concern of “weak bones.” The patient reports a facial deformity since early adolescence which necessitated debulking surgeries in the 1960s and in 2000. She reports left facial pain, neck pain, intermittent diplopia, and episodes of epistaxis dating back to 2010. She denies a history of fractures despite a diagnosis of osteoporosis. She denies a history of radiation treatment or endocrinopathies other than osteoporosis. She denies a family history of endocrine disorders. Physical examination revealed prominent leontiasis ossea and dysarthria secondary to a large firm mass in the proximity of her mandible (Fig. 1). Cranial nerves 1 through 12 were intact. Patient was edentulous, had a deviated nasal septum, and lateral superior displacement of the left eye. She had cervical kyphosis without scoliosis. Her calcium, phosphate, creatinine, 25-hydroxycholecalciferol, 1,25-dihydroxycholecalciferol, alkaline phosphatase, and C-terminal telopeptide were all within normal limits. A bone scan was remarkable for uptake involving her left facial bones. A maxillofacial computed tomography (CT) was performed as shown (Fig. 2). What is the diagnosis? Fig. 2 View Large Image Figure Viewer

Leontiasis Ossea Following Secondary Hyperparathyroidism and Hemodialysis

Leontiasis ossea is a rare medical condition which is characterized by an overgrowth of the facial and cranial bones secondary to chronic renal failure and secondary hyperparathyroidism. We reported a case of leontiasis ossea with history of secondary hyperparathyroidism due to end-stage renal disease on regular hemodialysis. A 37-year-old female with end-stage renal disease from lupus disease on regular hemodialysis complaining of progressive facial deformity for two years causing dyspnea, dental malocclusion and dysarthria with the final diagnosis of uremic leontiasis ossea. It is important to recognize features of leontiasis ossea, as it may result in life-threatening upper airway obstruction and compressive cranial neuropathy while after parathyroidectomy, facial changes can be stabilized or improved mildly.

CT and MR Imaging in a Large Series of Patients with Craniofacial Fibrous Dysplasia.

SummaryBackgroundIn this retrospective review of patients with craniofacial fibrous dysplasia (FD), the clinical and radiological findings of CT and MR scan were analyzed.Material/MethodsThe study material included 32 patients, at 9 to 68 years of age that were directed for differential diagnostics of several disorders in the head. We recorded CT and MRI data related to the lesion number, location, sidedness, appearance, and sex of the cases with craniofacial FD.ResultsOf 32 patients involved in this study, 17 had monostotic and 15 had polyostotic involvement pattern. Bones most commonly involved by monostotic involvement in females were, in descending order, mandibular, maxillary, and sphenoid bones, while the sphenoid bone was involved the most in males. Leontiasis ossea was observed in 2 patients. Sclerotic and mixed lesion types were more common in both females and males. In T1- and T2-weighted MRI sequences, hypointensity was more common compared to hyperintensity or heterogeneous intensity. The type of enhancement of lesions was found similar after contrast medium administration.ConclusionsIn the presence of craniofacial FD during CT or MRI imaging of the head, a detailed description of FD lesions may provide an important clinical benefit by increasing radiological experience during the diagnostics of this rare disorder.

Lhermitte-Duclos Disease: Diagnosis on MRI, MR Spectroscopy, CT and Positron Emission Tomography.

A 30-year-old male patient presented with progressive occipital headache since one year with no associated nausea, vomiting or gait disturbance. Neurological examination revealed negative Rhomberg’s sign. Computed tomography revealed non-enhancing hypodense mass in right cerebellar hemisphere compressing fourth ventricle with obstructive hydrocephalus, however it was inconclusive. Afterwards, MRI was performed which revealed right cerebellar mass with striated hypo and iso intense signal on T1W images, hyper and iso intense signal on T2W images. Mass was compressing fourth ventricle postero-inferiorly with obstructive hydrocephalus. Associated inferior tonsillar herniation into upper cervical canal was also noted [Table/Fig-1,​,22]. [Table/Fig-1]: T2W axial (A) & FLAIR (B) coronal images reveals right cerebellar mass (arrows) with alternate bands of hyperintense and isointense signal giving “tiger stripes” appearance with mass effect over cisterna magna & 4th ventricle ... [Table/Fig-2]: T1W sagittal image reveals alternating bands of relative isointense and hypointense signal within the lesion (arrow) with mild inferior tonsillar herniation Proton MRS showed reduced NAA, Choline peaks. Cho/Cr and NAA/Cr ratios were within normal limits [Table/Fig-3]. On Flurodeoxyglucose (FDG) Positron emission tomography (PET) and PET-CT examinations the mass lesion showed evidence of hypermetabolism [Table/Fig-4,​,55]. [Table/Fig-3]: MRS image reveals reduced peaks of NAA and choline, decreased Cho/Cr ratio (0.65). NAA/Cr (1.02) & NAA/Cho (0.94) ratios were also reduced compared to normal [Table/Fig-4]: PET reveals increased FDG uptake in the lesion suggesting hypermetabolism (arrow) [Table/Fig-5]: PET, CT reveals increased FDG uptake by the right cerebellar mass (arrow) Considering diagnosis of Lhermitte-Duclos disease, Ultrasound and CT-scan of chest and abdomen were done, but they did not reveal any abnormality hence ruling out features suggesting Cowden Syndrome. Patient was operated and avascular mass was resected with free margins and sent for histopathological examination. HPE revealed WHO grade-I dysplastic cerebellar gangliocytoma according to criteria for the diagnosis of Lhermitte-Duclos disease. Postoperative course was uneventful and patient was discharged within 1 week after surgery without any neurological deficit. First described in 1920 [1] by Lhermitte and Duclos, Lhermitte Duclos disease (LDD) (dysplastic gangliocytoma) is a rare disorder of uncertain pathogenesis characterized by disarrangement of normal cerebellar laminar cytoarchitexture. This entity has been described by variety of terms like granular cell hypertrophy, ganglioneuroma and gangliocytoma dysplasticum [2]. LDD is found to be associated with Cowden syndrome in 40% cases. Cowden syndrome is also known as multiple hamartoma syndromes, a rare autosomal dominant condition characterized by multiple hamartomas and neoplasms of ectodermal, mesodermal and endodermal origin [3]. LDD can also be accompanied by megalencephaly, hydromelia, heterotopia, microgyria, polydactyly, leontiasis ossea and multiple haemangiomas [2]. Our patient did not show any features of this syndrome. Although LDD usually occurs in third or fourth decades, reported age ranges from newborn to 74 years [3]. Clinically, LDD typically presents with findings of increased intracranial pressure in young adults. Many patients suffer from headache due to increased intracranial pressure caused by mass effect and obstructive hydrocephalus. Nausea, vomiting, visual disturbance and cerebello-vestibular symptoms can also be seen [3]. Classical MRI findings of LDD is described as especially unilateral, non enhancing hemispheric mass with layered appearance consisting of alternating bands of relative iso-, and hypointensity to normal brain on T1W, and relative hyper and iso intensity on T2W images [3]. Wolansky et al., mentioned that “tiger stripes” appearance is so distinctive that that it is unlikely to be confused with another entity. Non-enhancement is not a rule, since LDD lesions with contrast enhancement have been reported rarely [4]. Our case showed right cerebellar lesion with hemispheric expansion and typical striated pattern resembling tiger stripes on both T1- and T2W images with no contrast enhancement or perilesional oedema. MRS shows some characteristic findings such as decreased NAA with increased lactate peak. Lack of increased Cho/Cr ratio is striking which are in contrast to cerebral tumors where choline levels are raised. Our case showed decrease level of NAA and choline. Cho/Cr ratio was also decreased (0.65). NAA/Cr (1.02) & NAA/Cho (0.94) ratios were also reduced compared to normal. The disease is difficult to diagnose on CT, which shows poorly defined hypodense lesion with no contrast enhancement but may show calcification and thinning of occipital bone. FDG-PET revealed increased 18-FDG uptake within whole right cerebellar lesion suggesting either increased overall cell metabolism or an isolated up regulation in enzyme activity of hexokinase remains unclear. Histological examination reveals typical findings of thickening and hypermyelination of the molecular layer and large pleomorphic cells replacing purkinje and granular cell layers. Demyelination of central white matter of the folia is also observed. Conventional MRI shows characteristic and pathognomic features of LDD, however MRS can provide useful additional information regarding benign nature of this lesion. Hypermetabolism on FDG-PET further supports diagnostic accuracy. Thus imaging can achieve accurate preoperative diagnosis of LDD along with delineation of the margins, thereby enabling complete excision.

Uremic leontiasis ossea

Uremic leontiasis ossea is a rare manifestation of renal osteodystrophy clinically characterized by jaw enlargement, widening of the nares, flattening of the nasal bridge, and increased interdental spacing. Computed tomography (CT) findings are particular characteristic and include serpiginous tunneling within the maxillofacial bones and cortical bone resorption. Nuclear medicine scans are also useful for demonstrating hyperplasia of the parathyroid glands. Ultimately, the diagnosis of uremic leontiasis ossea can be made non-invasively through a combination of clinical parameters and imaging findings, as described in this article.

Computed tomography and magnetic resonance imaging of maxillofacial lesions in renal osteodystrophy.

This study aims to describe the computed tomography (CT) and magnetic resonance (MR) imaging appearance of maxillofacial lesions in renal osteodystrophy. We retrospectively reviewed the CT and MR imaging of maxillofacial region in 9 patients (6 females and 3 males with mean age of 31 yr) with renal osteodystrophy. They presented with facial swelling (n = 6), facial disfigurement (n = 2), and oral cavity mass (n = 1). They underwent CT and MR imaging of the maxillofacial region. Brown tumors (n = 6) were seen in the mandible (n = 4) and maxilla (n = 2). They appeared as mixed lytic and sclerotic (n = 4) and sclerotic (n = 2) lesions at CT. The lesions appeared as hypointense at T1-weighted images and of mixed signal intensity at T2-weighted images with intense contrast enhancement (n = 6). Uremic leontiasis ossea (n = 2) appeared at CT as diffuse hyperostosis with protruded maxilla and obliterated sinus. At MR imaging, there was expansion of the maxilla with obliteration of the maxillary sinuses and protrusion of the mandible. The lesion exhibited low signal intensity at T1-weighed images. At T2-weighted images, the lesion showed low signal intensity with small hyperintense lesions. Dystrophic calcification (n = 2) was seen in the parotid and the check. We concluded that CT and MR imaging are helpful for diagnosis and treatment planning of maxillofacial lesions of patients with renal osteodystrophy.

Total parathyroidectomy with autotransplantation for a rare disease derived from uremic secondary hyperparathyroidism, the uremic leontiasis ossea

We described six uremic leontiasis ossea (ULO) patients who underwent total parathyroidectomy with autotransplantation. ULO demonstrated more a systemic disease than a simple craniofacial deformation. The surgery seemed an effective treatment to alleviate secondary hyperparathyroidism and to improve patients' quality of life. ULO may have a high postoperative recurrence tendency. ULO is a rare disease derived from uremic secondary hyperparathyroidism (SHPT). Previous studies mostly focused on the craniofacial deformations. This study aims to investigate the systemic features of the disease and the surgical outcomes. The present study retrospectively assessed six ULO patients who underwent total parathyroidectomy (TPTX) with autotransplantation (AT). Follow-up data were recorded. The follow-up status was considered as "effectiveness" if serum intact parathyroid hormone (iPTH) levels were <150 pg/mL in the first 3 days after surgery, or as "recurrence" if serum iPTH gradually increased >300 pg/mL during follow-up in patients whose status was initially considered as "effectiveness". Craniofacial deformations, short stature, thoracocyllosis, spine malformations, osteodynia, and muscle weakness were observed in all patients. Abnormal pulmonary functions were observed in five patients. After surgery, one patient died from respiratory failure. Surgery was effective in the remaining five patients with relieved osteodynia and stopped craniofacial deformation. A mean follow-up of 7.6 (4 to 12) months was available. Three patients suffered from recurrence of hyperparathyroidism originating from autografts. Our data suggests that ULO is not only a simple disease with craniofacial malformations but is a severe systemic disease leading to increased surgical risks. TPTX with AT seems an effective treatment to relieve SHPT and to improve quality of life. ULO may have a high postoperative recurrence tendency.